目的

探讨儿童原发性干燥综合征（pSS）的临床诊治特点及早期肾损害情况。

方法

选择2017年1月至2023年1月在首都医科大学附属首都儿童医学中心风湿免疫科住院治疗的30例儿童pSS患儿为研究对象。回顾性分析其一般临床资料、实验室检查结果、肾损伤情况及治疗、转归、预后。本研究遵循的程序符合2013 年修订的《世界医学会赫尔辛基宣言》要求。

结果

①这30例pSS患儿中，男、女性患儿比例为1∶5，起病年龄及确诊年龄分别为（8.9±3.8）岁和（10.3±3.4）岁；系统受累主要临床症状为咽扁桃体反复肿大/反复腮腺炎（23/30，76.7%），血液系统受累（17/30，56.7%），局限性皮肤血管炎（12/30，40.0%），多形红斑（2/30，6.7%），弥漫性皮肤血管炎（1/30，3.3%）。②实验室检查结果显示，14例（14/30，46.7%）唇腺活组织检查示腺体萎缩，间质多灶性淋巴浸润，每灶淋巴细胞数＞50个；3例（10.0%）接受腮腺造影结果显示末梢导管斑点至小球状扩张，排空不全。③30例pSS患儿干燥综合征疾病活动指数（ESSDAI）评分为（14.7±7.8）分，干燥综合征疾病活动度（SSDA）为中至重度。30例患儿均未累及神经系统。其中，伴血清学结果变化为24例（80.0%），腺样体病变为23例（76.7%），血液系统病变为17例（56.7%），主要表现为血小板计数降低，伴不同程度皮肤病变为15例（50.0%）。④30例pSS患儿中，5例（16.7%）尿蛋白呈弱阳性至阳性（±～+），4例（13.3%）患儿24 h尿蛋白定量异常升高（153.7～787.8 mg/d）。pSS患儿早期肾损伤发生率较高（90.9%，20/22），其中早期肾小管损伤发生率最高（86.4%，19/22），但是程度较轻；早期肾小球损伤发生率为31.8%（7/22）。⑤对30例pSS患儿的治疗以口服激素、免疫抑制剂及硫酸羟氯喹片（HCQ）为主。对其中23例患儿的随访时间为17个月（7个月，38个月），随访结果显示21例患者临床症状稳定，炎性指标正常，激素规律减量过程中无病情反复。

结论

儿童pSS主要临床表现及受累系统为咽扁桃体反复肿大/反复腮腺炎，其次为血液系统受累。反复腮腺炎症状应作为儿童pSS的诊断标准之一。pSS患儿经积极治疗后，多数可于随访期间临床症状稳定。儿童pSS的早期肾损伤发生率较高，特别是肾小管损伤发生率较高，pSS患儿应积极完善肾穿刺活组织检查。

Objective

To investigate the clinical manifestations and early renal involvement in pediatric patients with primary Sjögren's syndrome （pSS）.

Methods

A total of 30 pediatric patients with pSS who were hospitalized in the Department of Rheumatology and Immunology，Capital Center for Children's Health，Capital Medical University，between January 2017 and January 2023 were included in this study.A retrospective analysis was conducted on their general clinical data，laboratory test results，renal involvement，and prognosis.This study followed the procedures in accordance with the DeclarationofHelsinkioftheWorldMedicalAssociation revised in 2013.

Results

①Among the 30 children with pSS，the male-to-female ratio was 1∶5.The age of onset and the age at diagnosis were （8.9±3.8）years and （10.3±3.4）years respectively.The predominant systemic manifestations included recurrent glandular swelling or parotitis （23/30，76.7%），hematologic involvement（17/30，56.7%），localized cutaneous vasculitis （12/30，40.0%），erythema multiforme （2/30，6.7%），and diffuse cutaneous vasculitis （1/30，3.3%）.②Laboratory examinations revealed that among 30 cases，14 cases（46.7%）showed labial salivary gland atrophy with multifocal lymphocytic infiltration in the interstitium，and the number of lymphocytes in each focus exceeded 50.Additionally，3 cases（10.0%）underwent sialography，which demonstrated punctate to small saccular dilation of the terminal ducts with incomplete emptying.③Among the 30 children with pSS，the mean European League Against Rheumatism （ESSDAI）score was 14.7±7.8，indicating moderate to severe disease activity.None of the children had neurological involvement.Serum abnormalities were present in 24 cases （80.0%），glandular involvement in 23 cases （76.7%），and hematologic involvement in 17 cases （56.7%），with thrombocytopenia being the predominant manifestation.Skin lesions of varying degrees were observed in 15 cases （50.0%）.Among the 30 children with pSS，5 cases（16.7%）exhibited trace to mild proteinuria（±to+），and 4 cases （13.3%）had abnormal elevation in 24 h urinary protein excretion （153.7～787.8 mg/d）.The prevalence of early renal involvement in children with pSS was relatively high （90.9%，20/22），with a higher incidence of early tubular injury（86.4%，19/22），although the severity was mild.The incidence of early glomerular injury was 31.8%（7/22）.The treatment for the 30 children with pSS primarily consisted of oral corticosteroids，immunosuppressive agents，and hydroxychloroquine sulfate （HCQ）.Among them，23 children were followed up for a median duration of 17 months （ranging from 7 to 38 months）.During this period，21 patients achieved stable clinical symptoms，normalized inflammatory markers，and no disease relapse was observed during the gradual tapering of corticosteroids.

Conclusions

The primary clinical manifestations and affected systems in pediatric pSS include recurrent glandular swelling/repeated parotitis，followed by hematologic involvement.Recurrent parotitis should be considered as one of the diagnostic criteria for pediatric pSS.With active treatment，most children with pSS achieve stable clinical symptoms during follow-up.The incidence of early renal involvement in pediatric pSS is relatively high，especially the rate of renal tubular injury.Renal biopsy should be actively performed in children with pSS.